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Abramson Cancer Center
NCI CANCERLIT® Search: Soft Tissue Sarcoma/Rhabdomyosarcoma - March 2002
National Cancer Institute®
Last Modified: March 1, 2002
Table of Contents
1
UI - 11642491
AU - Shields JA; Shields CL; Brotman HK; Carvalho C; Perez N; Eagle RC Jr
TI -
Cancer metastatic to the orbit: the 2000 Robert M. Curts Lecture.
SO - Ophthal Plast Reconstr Surg 2001 Sep;17(5):346-54
AD - Oncology Service, Wills Eye Hospital, Thomas Jefferson University,
Philadelphia, Pennsylvania 19107, USA.
PURPOSE: To report the demographics and clinical features of a large
series of patients with orbital metastasis. METHODS: Retrospective chart
review on 100 consecutive patients and a literature review on orbital
metastasis. RESULTS: Of 100 patients, the primary tumor site was breast
in 53 (53%), prostate gland in 12 (12%), lung in 8 (8%), skin (melanoma)
in 6 (6%), kidney in 5 (5%), gastrointestinal tract in 5 (5%), choroid
(melanoma) in 2 (2%), parotid gland in 1 (1%), and adrenal gland
(neuroblastoma) in 1 (1%). Of patients in whom a detailed history was
available, there was no history of cancer at the time of presentation in
19%. In 10%, the primary tumor remained undetected despite systemic
evaluation. There were 36 male patients and 64 female patients whose
mean age at diagnosis was 62 years (median 60 years, range 5 to 91
years). Both the right and left orbits were affected equally, and 4
cases (4%) were bilateral. The most frequent clinical findings were
limited ocular motility (54%), proptosis (50%), and palpable mass (43%).
The diagnoses were established by history, systemic survey, imaging
studies, and biopsy. Treatment included chemotherapy, hormone therapy,
irradiation, surgical excision, or observation, depending on clinical
circumstances. Among patients with sufficient follow-up, 95% died of
metastasis, with overall mean survival of 15 months (median 15 months;
range 3 to 96 months) after orbital diagnosis. CONCLUSIONS: The most
common primary cancers that metastasize to the orbit are breast,
prostate gland, and lung cancer. In 19%, there is no history of cancer
when the patient presents with ophthalmic symptoms and in 10% the
primary site remains obscure despite systemic evaluation. The systemic
prognosis is generally poor.
2
UI - 11845358
AU - Unni KK
TI -
Cartilaginous lesions of bone.
SO - J Orthop Sci 2001;6(5):457-72
AD - Mayo Clinic, Department of Laboratory Medicine and Pathology, 200 First
Street SW, Rochester, MN 55905, USA.
Cartilaginous lesions of the skeleton are very unusual. It is extremely
important to correlate the roentgenographic features, the clinical
features, and the histological features to arrive at a definite
diagnosis. Most cartilaginous lesions are benign or of low-grade
malignancy. However, there are some subtypes of chondrosarcoma that
behave in a highly aggressive fashion.
3
UI - 11723748
AU - Otsuka T; Yonezawa M; Kamiyama F; Matsushita Y; Matsui N
TI -
Results of surgery and radio-hyperthermo-chemotherapy for patients with
soft-tissue sarcoma.
SO - Int J Clin Oncol 2001 Oct;6(5):253-8
AD - Department of Orthopaedic Surgery, Nagoya City University Medical
School, 1 Kawasumi, Mizuho-ku, Nagoya 467-8601, Japan.
t.otsuka@med.nagoya-cu.ac.jp
BACKGROUND: Between 1990 and 1999, we performed
radio-hyperthermo-chemotherapy (RHC) in 44 patients with high-grade
soft-tissue sarcomas of the limbs. METHODS: Radiotherapy involved the
delivery of radiation at a dose of 2 Gy once daily on 16 days, to give a
total dose of 32 Gy. Hyperthermia was conducted once a week, with a
total of five sessions. Chemotherapy was performed by implanting a
reservoir and administering cisplatin (3 mg/kg) three times, and
pinorubin (an adriamycin derivative; 1 mg/kg) twice by intra-arterial
infusion, at weekly intervals. These drugs were administered alternately
during hyperthermia sessions. RESULTS: Tumor shrinkage was observed in
98% (43/44) of the patients. Of the 36 patients with M0 tumors, 30 were
disease-free at final follow-up, 2 had no evidence of disease, 1 was
alive with disease, and 3 had died of the disease. Amputation was
required only in the first patient, and the affected limb was preserved
in the other 43 patients. The surgical margin was wide in 9 patients and
marginal in 29 patients, and intralesional excision was performed in 5
patients. There was recurrence in only 1 of the 44 patients. CONCLUSION:
RHC is currently the most potent and relatively safe treatment method
for high-grade soft-tissue sarcomas that is available clinically.
4
UI - 11870247
AU - Savage DG; Antman KH
TI -
Imatinib mesylate -- a new oral targeted therapy.
SO - N Engl J Med 2002 Feb 28;346(9):683-93
AD - Herbert Irving Comprehensive Cancer Center, Columbia University College
of Physicians and Surgeons, New York, NY, USA.
5
UI - 11843853
AU - Pandey M; Mathew A; Kattoor J; Abraham EK; Mathew BS; Rajan B; Nair KM
TI -
Malignant phyllodes tumor.
SO - Breast J 2001 Nov-Dec;7(6):411-6
AD - Division of Surgical Oncology, Regional Cancer Center, Medical College
PO, Thiruvananthapuram, Kerala, India.
The study aims to evaluate the survival and prognosis of patients with
malignant phyllodes tumor. Between 1982 and 1998, 37 women with
malignant phyllodes tumor were treated at the Regional Cancer Center,
Trivandrum. Twelve patients were recurrent. Survival was estimated using
the Kaplan-Meier method. Patient, disease, and treatment factors were
compared using log-rank test. The Cox-proportional hazard model was
employed to identify the prognostic factors. Thirty-six patients had
surgery. Twenty-five patients received postoperative radiotherapy, and 2
received chemotherapy in addition. The median follow-up was 43 months
(range 1-170 months). Eight patients failed locally, and 7 of these were
successfully salvaged by surgery. The 5-year overall survival was 74.2%
(95% CI, 0.44 to 0.89), whereas 5-year disease-free survival was 59.6%
(95% CI, 0.39 to 0.7). The margin of surgical excision was found to be
the only independent prognostic factor (p=0.003). However, patients with
tumor size more than 5 cm (hazard ratio 2.9) were found to have
increased hazard, whereas those receiving adjuvant radiotherapy (hazard
ratio 0.6), married women (hazard ratio 0.4), and those women over the
age of 35 years (hazard ratio 0.7) showed a decreased hazards.
Cystosarcoma phyllodes is a rare malignancy of the female breast.
Surgery with adequate margins is the primary treatment. Adjuvant
radiotherapy appears to improve the disease-free survival.
6
UI - 11823688
AU - Janjan N; Crane C; Delclos M; Ballo M
TI -
Brachytherapy for locally recurrent soft-tissue sarcoma.
SO - Am J Clin Oncol 2002 Feb;25(1):9-15
AD - University of Texas M. D. Anderson Cancer Center, 1515 Holcombe
Boulevard, Houston, TX 77030, U.S.A.
Radiation is used to reduce potential risk of local recurrence from
microscopic residual disease after surgical resection. Brachytherapy is
a clinically established means of providing radiation for soft-tissue
sarcomas that recur after surgical resection alone or surgical resection
and radiation. Although the total dose of radiation that is prescribed
is approximately the same for patients undergoing external beam
radiation or brachytherapy, the radiobiologic characteristics of
brachytherapy, based on the inverse-square law, provide higher doses of
radiation to the surgical bed. This provides a theoretical advantage for
the use of brachytherapy as compared with external beam radiation among
patients with recurrence after surgical resection. When soft-tissue
sarcomas recur in a previously irradiated area, further external beam
radiation generally is not possible; therefore, brachytherapy allows a
radiotherapeutic alternative in an attempt to reduce the risk of further
local recurrence. Recommendations for patient selection, the total dose
of radiation, and the radiation dose-rate are outlined. Standard grading
systems for response, symptoms, and severity of complications should be
used.
7
UI - 11856160
AU - Erhan Y; Zekioglu O; Ersoy O; Tugan D; Aydede H; Sakarya A; Kapkac M;
TI -
Ozdemir N; Ozbal O; Erhan Y
p53 and Ki-67 expression as prognostic factors in cystosarcoma
phyllodes.
SO - Breast J 2002 Jan-Feb;8(1):38-44
AD - General Surgery Department, Celal Bayar University, School of Medicine,
Guzelyali-Izmir, Turkey. ozeki@med.ege.edu.tr
We have reviewed the histopathological, clinical outcome and
immunohistochemical status in 21 women with cystosarcoma phyllodes (CSP)
tumors of the breast. We assessed 12 tumors as histopathologically
benign and 9 tumors as malignant. The median patient ages in benign and
malignant CSP tumors were 39.6 and 45.4 years of age, respectively. The
stromal cellularity, stromal cellular atypism, high mitotic activity,
atypic mitoses, stromal overgrowth, infiltrative tumor contour, and
heterologous stromal elements were significant features of the malignant
CSP tumors. Benign CSP tumors were predominantly of fibroadenomatous
architecture with cellular stroma (mild or moderate) and some distortion
and elongation of glandular elements. Five malignant CSP tumors were
stained positively with p53, and 6 malignant CSP tumors were stained
immunohistochemically with Ki-67. All benign CSP tumors were negatively
stained for p53 and Ki-67. The patients with benign CSP tumors were
treated with local excision ( n=11) and with subcutaneous mastectomy (
n=1). Malignant CSP tumors were treated with wide local excision ( n=1),
partial mastectomy ( n=1), simple mastectomy ( n=2), and modified
radical mastectomy ( n=5). Two patients with a high mitotic rate and
high values of p53 and Ki-67 received additional radiotherapy and
chemotherapy. One case had liver metastasis. This tumor had high mitotic
figures, stromal overgrowth, severe stromal cellularity, and 20% Ki-67
and mild p53 positivity. We suggest that p53 and Ki-67 can play an
important role in predicting prognosis and yielding additional therapy
besides conventional prognostic factors in the treatment of the CSP
patients.
8
UI - 11857314
AU - Edmonson JH; Petersen IA; Shives TC; Mahoney MR; Rock MG; Haddock MG;
TI -
Sim FH; Maples WJ; O'Connor MI; Gunderson LL; Foo ML; Pritchard DJ;
Buckner JC; Stafford SL
Chemotherapy, irradiation, and surgery for function-preserving therapy
of primary extremity soft tissue sarcomas: initial treatment with
ifosfamide, mitomycin, doxorubicin, and cisplatin plus granulocyte
macrophage-colony-stimulating factor.
SO - Cancer 2002 Feb 1;94(3):786-92
AD - Mayo Clinic, Rochester, Minnesota 55905, USA. edmoj@mayo.edu
BACKGROUND: Most institutional teams utilize multimodality therapy in
their efforts to cure patients with primary high-grade extremity soft
tissue sarcomas, although the value of adjuvant systemic chemotherapy is
still disputed by some oncologists. This single-institution Phase II
study describes an effort to control metastasis by the use of two cycles
of chemotherapy as the initial preoperative treatment. METHODS: Between
or limb girdle high-grade soft tissue sarcomas were registered to a
study of preoperative ifosfamide, mitomycin, doxorubicin, cisplatin
(IMAP) plus granulocyte macrophage-colony-stimulating factor (GM-CSF)
followed by preoperative irradiation and subsequent limb-sparing
surgery. The two sequential monthly cycles of IMAP involved intravenous
ifosfamide, 2500 mg/m(2), and mesna, 2500 mg/m(2), on Day 0, followed by
identical doses of these agents plus intravenous mitomycin, 4 mg/m(2),
doxorubicin, 40 mg/m(2), and cisplatin, 60 mg/m(2), on Day 1.
Sargramostim (GM-CSF) 250 microg/m(2) was given subcutaneously every 12
hours for 4 days beginning 6 days before the chemotherapy, and then for
14 more days beginning the day after chemotherapy was completed. At the
beginning of the third month, external beam irradiation was administered
daily, 5 days each week for 5 consecutive weeks to total preoperative
doses of 4500 centigrays (cGy). This was accompanied by reduced doses of
MAP chemotherapy (mitomycin, 6 mg/m(2), doxorubicin, 30 mg/m(2), and
cisplatin, 45 mg/m(2)) intravenously on Days 0, 21, and 42 of the
radiation therapy segment. Approximately 1 month after preoperative
irradiation ended, each patient had complete surgical excision with
curative intent, using limb-sparing techniques when possible. Radiation
to total doses of 5500-6500 cGy was accomplished by delivery of an
additional 1000-2000 cGy to the tumor bed via intraoperative electron
beam, brachytherapy, or external beam irradiation at the completion of
surgery. RESULTS: All except 5 patients had tumors at least 5 cm in
diameter. Chemotherapy toxicity grade three or higher consisted
primarily of vomiting (23%), leukopenia (54%), and thrombocytopenia
(77%). Six patients have died of metastatic sarcoma, and one other died
in a motorcycle accident. Kaplan-Meier curves indicate estimated 5-year
survival of approximately 80% and freedom from metastasis at 2 years of
approximately 85%. CONCLUSIONS: IMAP plus GM-CSF is satisfactory as
initial treatment for primary extremity soft tissue sarcomas in two
monthly cycles preceding irradiation. The prescribed irradiation was
generally tolerable and effective in permitting limb-sparing surgery.
Although the outcome of patients treated on this regimen has been
favorable, the metastasis problem has not been eliminated. Copyright
2002 American Cancer Society. DOI 10.1002/cncr.10259
9
UI - 11520418
AU - Cristiano K; Wirz M; Gentili G
TI -
Safety of Italian plasma for fractionation with respect to contamination
with human herpes virus 8.
SO - Vox Sang 2001 Jul;81(1):52-3
10
UI - 11872294
AU - Petersen IA; Haddock MG; Donohue JH; Nagorney DM; Grill JP; Sargent DJ;
TI -
Gunderson LL
Use of intraoperative electron beam radiotherapy in the management of
retroperitoneal soft tissue sarcomas.
SO - Int J Radiat Oncol Biol Phys 2002 Feb 1;52(2):469-75
AD - Division of Radiation Oncology, Mayo Clinic, Rochester, MN 55905, USA.
petersen.ivy@mayo.edu
PURPOSE: To evaluate the disease control, survival results, and
tolerance of intraoperative electron beam radiotherapy (IOERT) as a
component of treatment for retroperitoneal soft tissue sarcomas. METHODS
primary (n = 43) or recurrent (n = 44) retroperitoneal or intrapelvic
sarcomas received IOERT as a component of treatment at the Mayo Clinic.
The tumors were high grade in 54 patients (62%) and low grade in 33
(38%). The median tumor size was 10 cm (range 2-36). All patients
underwent maximal surgical resection with IOERT; in 72 patients, only
microscopic or no residual tumor remained. The IOERT doses ranged from
8.75 to 30 Gy (median 15). All primary tumors received external beam
irradiation (EBRT) with a median dose of 48.6 Gy. Thirty-four of the 44
recurrent tumors received EBRT to a median dose of 45 Gy. All patients
were followed prospectively for outcome and toxicity evaluation.
RESULTS: The median follow-up, based on 46 patients (53%) currently
alive, was 3.5 years. The overall estimated 5-year survival was 47%. For
patients with tumors > or = 10 cm, the 5-year overall survival was
significantly poorer (28%) than for those with smaller lesions (60%) (p
= 0.01). Neither primary vs. recurrent status nor tumor grade had a
significant impact on survival. Patients with gross residual tumor had a
marginally significantly poorer survival compared with patients with
microscopic or no residual tumor, with a 5-year survival rate of 37% and
52%, respectively (p = 0.08). A total of 49 patients (56%) experienced
failure, including 20 local recurrences (23%). The median time to
failure was 2.3 years. Four recurrences were within the IOERT field, 3
within the IOERT and EBRT field, and 13 within the EBRT field alone. The
3- and 5-year estimated local control rate was 77% and 59%,
respectively. Local control was marginally significantly affected by the
amount of residual tumor, with a 5-year local control rate of 41% for
those with gross residual tumor, 60% for those with microscopic residual
tumor, and 100% for those with no residual tumor (p = 0.09).
Gastrointestinal complications were recorded in 12 incidences (Grade 3
or higher toxicity). These complications were believed to be secondary
to surgery and/or EBRT in 10 of the 12 cases. Seven patients had fistula
formation, and 3 experienced severe proctitis. Grade 3 peripheral
neurologic toxicities occurred in 9 patients (10%), but none had pain as
a component of their neuropathy. CONCLUSION: Retroperitoneal soft tissue
sarcomas can be treated with an aggressive combined approach of EBRT,
surgery, and IOERT, with acceptable toxicity. Local control in primary
disease appears to be improved in this retrospective series with this
approach. Distant disease control and options for recurrent disease
needs further definition.
11
UI - 11776610
AU - Bai P; Zhang W; Sun J
TI -
[Combination chemotherapy of uterine sarcomas]
SO - Zhonghua Zhong Liu Za Zhi 2000 Jan;22(1):80-2
AD - Cancer Institute (Hospital), Chinese Academy of Medical Sciences, Peking
Union Medical College, Beijing 100021, China.
OBJECTIVE: To study the results of combination chemotherapy of uterine
sarcoma after operation and recurrent tumor. METHODS: One hundred
seventy-four cases of three major pathological subtypes of uterine
sarcomas were treated in the Cancer Hospital from 1960 to 1996. Clinical
data were analyzed of 51 cases of uterine sarcomas treated with
postoperative adjuvant chemotherapy and 38 cases with recurrent tumors
received 98 courses of chemotherapy. They were divided into 4 groups
according to the adjuvant chemotherapy regimen: single drug, VAC, VAD,
and other regimens. Chemotherapy regimens for recurrent tumors were VAD,
PA/PAC, and other combination regimens including etoposide, ifosfamide,
cisplatin, adriamycin. RESULTS: The 5-year survival rate of stage I-II
uterine sarcoma patients was 54.9% receiving adjuvant chemotherapy. It
was 72.7% in VAD group which was significantly higher than that in other
regimen groups. The survival rate was related to the number of
chemotherapy course. The chemo-sensitivity of various pathological types
of recurrent uterine sarcomas was not different. CONCLUSION: The 5-year
survival rate does not improve in patients with stage I-II uterine
sarcomas given postoperative chemotherapy. VAD is among the best
regimens and at least 3 courses should be performed. The results of new
treatment regimens such as EPA, IA, etc., must await further clinical
observation.
12
UI - 11828318
AU - Takanami I; Imamura T
TI -
The treatment of Askin tumor: results of two cases.
SO - J Thorac Cardiovasc Surg 2002 Feb;123(2):391-2
AD - Department of Surgery, Teikyo School of Medicine, Tokyo, Japan.
takanami@med.teikyo-u.ac.jp
13
UI - 11848531
AU - Schwarzbach MH; Dimitrakopoulou-Strauss A; Mechtersheimer G; Hinz U;
TI -
Willeke F; Cardona S; Attigah N; Strauss LG; Herfarth C; Lehnert T
Assessment of soft tissue lesions suspicious for liposarcoma by
F18-deoxyglucose (FDG) positron emission tomography (PET).
SO - Anticancer Res 2001 Sep-Oct;21(5):3609-14
AD - Department of Surgery, University of Heidelberg, Germany.
matthias_schwarzbach@med.uni-heidelberg.de
BACKGROUND: F18-deoxyglucose (FDG) positron emission tomography (PET) is
a promising imaging technique. The aim of this study was to investigate
the use of FDG PET in patients with suspected liposarcomas (LS).
PATIENTS AND METHODS: Forty-two masses were studied. The FDG uptake was
estimated in tumor (T) and normal tissue (NT). The data were analyzed
with respect to pathological findings. RESULTS: Pathology revealed 11
primary LS, 14 locally recurrent LS, 5 other sarcomas, 1 inflammation, 1
lymphoma and 10 benign lesions. FDG uptake (T-to-NT ratio) in 25 LS
corresponded with the histological subtype. Pleomorphic, mixed and
myxoid LS showed an increased T-to-NT ratio and were thus visualized.
Four out of six well-differentiated LS presented a low FDG uptake. Like
subtype, the tumor grade also corresponded to FDG uptake. The T-to-NT
ratio of higher grade LS, contrary to low grade LS, was uniformly
increased. Primary LS were distinguishable from benign tumors, while
other sarcomas, inflammation and lymphoma were not. Recurrence was
detected with a sensitivity of 86% and a specificity of 100%.
False-negative diagnoses occurred only in well-differentiated
recurrences. CONCLUSION: FDG uptake in LS correlates with the
histological subtype and tumor grade. The diagnostic value of FDG PET in
LS, therefore, is influenced by histomorphological parameters. Our data
suggest that pleomorphic, mixed and higher-grade LS recurrences are
preferentially amenable to FDG PET imaging.
14
UI - 11869019
AU - Kunisada T; Ngan SY; Powell G; Choong PF
TI -
Wound complications following pre-operative radiotherapy for soft tissue
sarcoma.
SO - Eur J Surg Oncol 2002 Feb;28(1):75-9
AD - Bone and Soft Tissue Sarcoma Unit, St. Vincent's Hospital and Peter
MacCallum Cancer Institute, Level 3 Daly Wing, 41 Victoria Parade,
Fitzroy, Victoria 3065, Australia.
AIMS: We analysed wound complications in 43 patients with soft tissue
sarcoma who were treated with combined pre-operative radiotherapy and
surgery. METHODS: All patients received the same protocol of
pre-operative radiotherapy at our institution. RESULTS: Thirty-six (84%)
patients developed acute skin toxicity following radiotherapy. After
wide local excision, 15 patients required primary soft tissue
reconstruction with vascularized muscle transfer and four patients
underwent free skin flap to enable wound closure as part of their
primary surgery. Nineteen patients (44%) developed post-operative wound
complications including 10 (23%) patients who required an additional
surgical procedure. Four (27%) patients developed flap necrosis in a
group of 15 who underwent primary vascularized soft tissue transfer. All
required a second vascularized muscular flap. One elderly patient, who
had grade 3 acute radiation skin toxicity, had an arterial graft and
total hip arthroplasty for a femoral artery aneurysm and an avascular
necrosis of the hip, respectively. In our series, age (> or = 40 years)
was the only impact factor influencing wound complication after surgery
following radiotherapy (P=0.06). CONCLUSIONS: Site of tumour, radiation
field size, surgical resection volume, grade of acute radiation
toxicity, co-morbidity, and smoking were not demonstrated to have
predictive value in wound complication following pre-operative
radiotherapy. Although previous papers suggested that vascularized soft
tissue transfer could be useful reducing wound morbidity, our results
could not confirm this. Copyright Harcourt Publishers Limited.
15
UI - 11869020
AU - Begossi G; Gonzalez-Moreno S; Ortega-Perez G; Fon LJ; Sugarbaker PH
TI -
Cytoreduction and intraperitoneal chemotherapy for the management of
peritoneal carcinomatosis, sarcomatosis and mesothelioma.
SO - Eur J Surg Oncol 2002 Feb;28(1):80-7
AD - The Washington Cancer Institute, Washington Hospital Center, Washington,
DC 20005, USA.
Despite new developments in multi-modality treatments, complete
resection remains as an absolute requirement for cure of
gastrointestinal cancer. We have reported benefits from combined
treatment with complete cytoreduction and intraperitoneal chemotherapy.
This has been achieved with low morbidity and mortality. Success in the
surgical management of peritoneal surface malignancy depends on the
surgeon's ability to complete complex cytoreductive procedures so that
only microscopic residual disease remains. This paper describes the
current strategy that the surgical oncologist should pursue in the
treatment of patients with peritoneal carcinomatosis, sarcomatosis and
mesothelioma. Technical details required for this surgery include
patient position, incision and exposure, complete lysis of adhesion,
electroevaporative dissection with irrigation and suction to preserve
the translucent quality of tissues, peritonectomy procedures, proper
positioning of tubes and drains for intraperitoneal chemotherapy, and
reconstructive surgery. Understanding the treatment and mastery of
surgical skills to manage the peritoneal surface spread of cancer has
led to long-term survival of selected patients. Combination of this
treatment strategy with proper patient selection has reduced the
mortality and morbidity. The success of cytoreductive surgery and
perioperative intraperitoneal chemotherapy depends on a long-term
dedication to achieve the full potential of a curative outcome. Our unit
has continued to achieve good results over two decades as improved
results of treatment have evolved. Copyright Harcourt Publishers
Limited.
16
UI - 11796846
AU - Nuciforo PG; O'Hara CD
TI -
Correspondence re: Bonetti F, Martignoni G, Colato C, Manfrin E,
Gambacorta M, Faleri M, et al. Abdominopelvic sarcoma of perivascular
epithelioid cells. Report of four cases in young women, one with
tuberous sclerosis. Mod Pathol 2001;14:563-8. And Tazelaar HD, Batts KP,
Srigley JR. Primary extrapulmonary sugar tumor (PEST): a report of four
cases. Mod Pathol 2001;14:615-22.
SO - Mod Pathol 2002 Jan;15(1):87-90
17
UI - 11815866
AU - Wu SS; Collins MH; de Chadarevian JP
TI -
Study of the regression process in cardiac rhabdomyomas.
SO - Pediatr Dev Pathol 2002 Jan-Feb;5(1):29-36
AD - Department of Pathology and Laboratory Medicine, MCP Hannemann
University School of Medicine and St. Christopher's Hospital for
Children, Front Street at Erie Avenue, Philadelphia, PA 19134, USA.
Rhabdomyomas are the most common primary cardiac tumors in children, and
have been shown to undergo spontaneous regression. The aim of our study
was to investigate morphologically and immunohistochemically some
mechanisms that may explain this clinical phenomenon. Eleven tumors from
three term newborn girls who had physical and radiographic features
pathognomonic of tuberous sclerosis were evaluated. Control specimens
were left and right heart sections from five autopsies of age- and
sex-matched patients who died of causes unrelated to the cardiovascular
system. The tumors had been surgically excised from various regions in
the heart, and all had similar "typical" histology. Histomorphologic
evaluation with von Kossa and alizarin-red stains and terminal
deoxynucleotidyl transferase-mediated deoxyuridine triphosphate nick
end-labeling (TUNEL) method were performed to evaluate cell
calcifications, necrosis, and apoptosis. Ubiquitin immunohistochemical
study was also conducted to evaluate intracytoplasmic protein
degradation. In cardiac rhabdomyomas (CR), all myocytes with acidophilic
cytoplasmic myofibrils showed strong intracytoplasmic ubiquitin
immunoreactivity, compared with the occasional weak cytoplasmic and
focal nuclear positivity in control heart sections. Calcified myocyte
nuclei were commonly seen in CR tumoral and nontumoral rhabdomyocytes,
whereas control nontumoral cardiac myocytes did not show any
calcification. The incidence of TUNEL reactivity seen in CR (4.8 nuclei
per 100 cardiac rhabdomyocyte nuclei) was higher than that seen in
control heart sections (1.7 nuclei per 106 cardiac myocytes, P < 0.005).
The data led us to conclude that the cytoplasmic contents in CR were
degraded via the ubiquitin pathway, and from our observation of
increased TUNEL positivity, the rate of cell death in CR appeared
increased. These findings may explain, to some extent, the mechanism of
tumor regression.
18
UI - 11276029
AU - Polgar C; Orosz Z; Fodor J
TI -
Is postirradiation angiosarcoma of the breast so rare and does breast
lymphedema contribute to its development?
SO - J Surg Oncol 2001 Mar;76(3):239-41
19
UI - 11836422
AU - Lagunoff M; Bechtel J; Venetsanakos E; Roy AM; Abbey N; Herndier B;
TI -
McMahon M; Ganem D
De novo infection and serial transmission of Kaposi's sarcoma-associated
herpesvirus in cultured endothelial cells.
SO - J Virol 2002 Mar;76(5):2440-8
AD - Department of Microbiology and Medicine, Howard Hughes Medical
Institute, University of California-San Francisco, 513 Parnassus Ave.,
San Francisco, CA 94143-0414, USA.
Infection by Kaposi's sarcoma-associated herpesvirus (KSHV) is central
to the pathogenesis of the endothelial neoplasm Kaposi's sarcoma (KS)
and is also linked to the rare B-cell tumor known as primary effusion
lymphoma (PEL). Latently infected PEL cell lines can be induced to enter
the lytic cycle and produce KSHV virions. However, such cells do not
support de novo infection or serial propagation of KSHV. These
limitations have prevented the development of systems for the genetic
analysis of KSHV and have impeded a deeper understanding of KS
pathogenesis. Here we show that human dermal microvascular endothelial
cells immortalized by expression of telomerase can be readily infected
by KSHV virions produced by PEL cells. Infection is predominantly
latent, but a small subpopulation enters the lytic cycle spontaneously.
Phorbol ester (tetradecanoyl phorbol acetate [TPA]) treatment of
latently infected cells leads to enhanced induction of lytic KSHV
replication, resulting in foci of cytopathic effect. There is no
cytopathic effect or viral DNA expansion when infected TIME cells
(telomerase-immortalized microvascular endothelial cells) are TPA
induced in the presence of phosphonoacetic acid (PAA), an inhibitor of
herpesvirus replication. Supernatants from phorbol-induced cultures
transfer latent KSHV infection to uninfected cells, which can likewise
be induced to undergo lytic replication by TPA treatment, and the virus
can be further serially transmitted. Serial passage of the virus in TIME
cells is completely inhibited when TPA treatment is done in the presence
of PAA. Latently infected endothelial cells do not undergo major
morphological changes or growth transformation, and infection is lost
from the culture upon serial passage. This behavior faithfully
recapitulates the behavior of spindle cells explanted from primary KS
biopsies, strongly supporting the biological relevance of this culture
system. These findings suggest that either the stability or the
growth-deregulatory potential of the KSHV latency program in endothelial
cells is more limited than might be predicted by analogy with other
oncogenic viruses.
20
UI - 11443456
AU - Nakajima J; Takamoto S; Tanaka M; Takeuchi E; Murakawa T; Fukami T
TI -
Thoracoscopic surgery and conventional open thoracotomy in metastatic
lung cancer.
SO - Surg Endosc 2001 Aug;15(8):849-53
AD - Department of Cardiothoracic Surgery, Faculty of Medicine, University of
Tokyo, 7-3-1, Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
nakajima-tho@h.u-tokyo.ac.jp
BACKGROUND: We performed a retrospective comparison of the oncological
outcome of thoracoscopic surgery for pulmonary metastasis with that of
conventional open thoracotomy. METHODS: The patient population for our
retrospective comparison was comprised of 45 patients undergoing
pulmonary resections via video-assisted thoracoscopy (thoracoscopy
group) and 55 undergoing similar resections by open thoracotomy (open
group) for pulmonary metastases between 1994 and 1999. RESULTS: Solitary
metastasis was resected more frequently with thoracoscopy than open
thoracotomy. There were no significant intergroup differences in rates
of local recurrence from the initial pulmonary resection site. The
actuarial 1-year, 2-year, and 3-year survival rates were, respectively,
82.8%, 70.0%, and 62.3% in the thoracoscopy group and 93.6%, 64.6%, and
52.7% in the open group. The rates of pulmonary recurrence and survival
also did not differ significantly between the two groups with solitary
metastases. CONCLUSION: Thoracoscopic surgery for metastatic lung
disease appears to be feasible as long as the preoperative metastatic
tumor evaluation using chest computed tomography (CT) is accurate.
21
UI - 11786573
AU - Ferrari A; Bisogno G; Casanova M; Meazza C; Piva L; Cecchetto G; Zanetti
TI -
I; Pilz T; Mattke A; Treuner J; Carli M
Paratesticular rhabdomyosarcoma: report from the Italian and German
Cooperative Group.
SO - J Clin Oncol 2002 Jan 15;20(2):449-55
AD - Pediatric Oncology and Pediatric Surgery Units, Istituto Nazionale
Tumori, Milan, Italy. ferrari@istitutotumori.mi.it
PURPOSE: We report the experience of the German-Italian Cooperative
Group with 216 pediatric patients with paratesticular rhabdomyosarcoma
treated over 20 years. PATIENTS AND METHODS: At diagnosis, 198 patients
had localized disease and 18 had distant metastases. Among the
nonmetastatic patients, complete tumor resection was performed in 83% of
cases. Evaluation of the retroperitoneal lymph nodes changed over the
years from routine surgical staging to radiologic assessment. All
patients received chemotherapy, which was reduced in intensity and
duration for patients with low-risk features in subsequent protocols.
Radiotherapy was administered to 10% of patients. RESULTS: Among 72
patients with a negative retroperitoneal computed tomography (CT) scan,
surgical assessment detected nodal involvement in only one case. Among
23 patients with enlarged nodes on CT scans, surgery confirmed nodal
spread in 65% of patients. No differences in the rate of nodal
involvement were observed over the years. With a median follow-up of 110
months, 5-year survival was 85.5% for the series as a whole, 94.6% for
patients with localized disease, and 22.2% for metastatic cases.
Retroperitoneal nodal recurrence was the major cause of treatment
failure. Univariate analysis revealed the prognostic value of tumor
invasiveness, size, and resectability, as well as of nodal involvement
and age, in patients with localized tumor. CONCLUSION: The outcome for
patients with localized paratesticular rhabdomyosarcoma is excellent,
despite the reduction in chemotherapy over the years: an alkylating
agent-free and anthracycline-free regimen is adequate treatment for
low-risk patients. Surgical assessment of the retroperitoneum must be
reserved for patients with enlarged nodes on CT scans. Children over 10
years old carry a higher risk of nodal involvement and relapse.
22
UI - 11868476
AU - Palacios E; Friedlander PL
TI -
Chondrosarcoma of the larynx.
SO - Ear Nose Throat J 2002 Feb;81(2):83
AD - Department of Radiology, Louisiana State University Health Sciences
Center, New Orleans, USA.
23
UI - 11886321
AU - Andreoni M; Sarmati L; Nicastri E; El Sawaf G; El Zalabani M; Uccella I;
TI -
Bugarini R; Parisi SG; Rezza G
Primary human herpesvirus 8 infection in immunocompetent children.
SO - JAMA 2002 Mar 13;287(10):1295-300
AD - Department of Public Health and Cellular Biology, University Tor
Vergata, Via Montpellier 1, 00133 Rome, Italy. andreoni@uniroma2.it
CONTEXT: Human herpesvirus 8 (HHV-8) infection causes Kaposi sarcoma and
lymphoproliferative disorders in immunosuppressed adults. Its
manifestations in immunocompetent hosts are unknown. OBJECTIVES: To
determine whether HHV-8 primary infection is symptomatic in
immunocompetent children and to identify the epidemiological and
virological correlates of HHV-8 infection. DESIGN AND SETTING:
Prospective cohort study conducted in the pediatric emergency department
of a hospital in Alexandria, Egypt, between December 1, 1999, and April
30, 2000. PATIENTS: Eighty-six children aged 1 to 4 years who were
evaluated for a febrile syndrome of undetermined origin. MAIN OUTCOME
MEASURES: Serological assay and polymerase chain reaction of blood and
saliva samples for HHV-8. Information on potential risk factors for
HHV-8 infection was also collected. RESULTS: Thirty-six children (41.9%)
were seropositive; HHV-8 DNA sequences were detected in 14 (38.9%) of
these 36 children (detected in saliva in 11 of 14). Significant
associations were found between HHV-8 infection and close contact with
at least 2 other children in the community (36 of 63 vs 6 of 23 for <2
children; adjusted odds ratio [OR], 3.50; 95% confidence interval [CI],
1.11-12.22) and admission to the emergency department in December or
January (28 of 47 vs 14 of 39 for February-April; adjusted OR, 3.15; 95%
CI, 1.23-8.58). Six children had suspected primary HHV-8 infection; all
but 1 had a febrile cutaneous craniocaudal maculopapular rash, which was
more common among these children (5 of 6 vs 10 of 75; P<.001). For 3 of
these 6 children, a second blood sample was obtained after the
convalescence phase, and all 3 seroconverted for HHV-8. CONCLUSIONS:
Primary infection with HHV-8 may be associated with a febrile
maculopapular skin rash among immunocompetent children. The finding of
HHV-8 DNA sequences in saliva supports the hypothesis that transmission
through saliva is the main mode of transmission in the pediatric age
group.
24
UI - 11850069
AU - Nishio J; Iwasaki H; Ohjimi Y; Ishiguro M; Isayama T; Naito M; Iwashita
TI -
A; Kikuchi M
Overrepresentation of 17q22-qter and 22q13 in dermatofibrosarcoma
protuberans but not in dermatofibroma: a comparative genomic
hybridization study.
SO - Cancer Genet Cytogenet 2002 Jan 15;132(2):102-8
AD - Department of Pathology, School of Medicine, Fukuoka University,
Fukuoka, Japan. nishio@minf.med.fukuoka-u.ac.jp
Histopathological differentiation between dermatofibrosarcoma
protuberans (DFSP) and dermatofibroma (DF) is often difficult, because
both neoplasms share some clinical features and the presence of a
storiform pattern. In the present study, we investigated the usefulness
of comparative genomic hybridization (CGH) in the diagnosis of these
entities by examining 12 DFSP and 12 DF cases. The most frequent DNA
sequence copy number changes detected in 10 (83%) of 12 DFSP cases
(mean, 1.9 aberrations/tumor; range, 0-3) consisted of gains of
17q22-qter (10 tumors), 22q13 (nine tumors), and 8q24.1-qter (three
tumors). High-level amplification, which was detected in three tumors,
was seen only in chromosome 17, with 17q23-q25 as the minimal common
region. Loss of DNA sequences was not found in DFSP cases. In contrast,
two (17%) of the 12 DF cases (mean, 0.5 aberrations/tumor; range, 0-4)
showed DNA sequence copy number changes, although recurrent gains and
losses and high-level amplifications were not observed. Gains were more
common than losses in DF. Overrepresentation of 17q and 22q sequences
was a common finding in DFSP but not in DF. Thus, CGH seems to be useful
for distinguishing DFSP from DF in most cases.
25
UI - 11850075
AU - Mathew S; Dalton J; Riedley S; Spunt SL; Hill DA
TI -
Complex t(X;18)(p11.2;q11.2) with a pericentric inversion of the X
chromosome in an adolescent boy with synovial sarcoma.
SO - Cancer Genet Cytogenet 2002 Jan 15;132(2):136-40
AD - Department of Pathology, St. Jude Children's Research Hospital, 332
North Lauderdale, Memphis, TN 38105-2794, USA. susan.mathew@stjude.org
Synovial sarcoma is the most common nonrhabdomyosarcomatous soft-tissue
sarcoma in children and young adults. It is characterized by the common
t(X;18)(p11.2;q11.2) that results in the fusion of SYT on chromosome 18
to one of two closely related and adjacent genes on the X chromosome,
SSX1 or SSX2. Here we describe a poorly differentiated, monophasic
synovial sarcoma in a 17-year-old adolescent boy. Hyperdiploidy, a
t(X;18)(q13;q11), and other structural abnormalities were detected by
conventional cytogenetic analysis. Fluorescence in situ hybridization
with the PAC probe RP3-519N18, which is specific for the Xp11 region,
resulted in a signal on the der(Xq), a finding consistent with a
pericentric inversion of the X chromosome that resulted in a
t(X;18)(p11.2;q11.2)inv(X)(p11.2q13). Real-time polymerase chain
reaction using primer sets specific for SYT-SSX1 and SYT-SSX2 confirmed
the presence of an SYT-SSX1 fusion transcript. Our finding of this
unique and complex translocation in synovial sarcoma demonstrates the
utility of molecular methods in confirming the diagnosis of synovial
sarcoma.
26
UI - 11700885
AU - Montironi R
TI -
Spectrum of prostatic non-epithelial tumour-like conditions and tumours.
SO - Pathol Res Pract 2001;197(10):653-5
27
UI - 11847031
AU - Bui BN; Tabrizi R; Dagada C; Trufflandier N; St ckle E; Coindre JM
TI -
[Update on soft tissue sarcomas]
SO - Bull Cancer 2002 Jan;89(1):100-7
AD - Institut Bergonie, Centre regional de lutte contre le cancer, 229, cours
de l'Argonne, 33076 Bordeaux Cedex.
Important refinements have taken place in the diagnosis of soft tissue
sarcoma with extensive use of immuno-histochemistry. New entities have
been described, while malignant histiocytofibroma, the most diagnosed
sarcoma type during the last two decades, has been dismembered. As for
prognosis, the new UICC classification is effectively more
discriminating in the definition of prognostic groups; but the
usefullness of new biological or genetic markers remains to be assessed.
Several breakthrough have taken place in the last years in the treatment
of soft tissue sarcoma. Isolated limb perfusion with TNF, hyperthermia
and melphalan have proven its efficacy, and is now an alternative to
preoperative chemotherapy and/or radiotherapy for limb sparing treatment
of the primary tumor site or to amputation. For systemic treatments,
novel cytostatic drugs have been shown to be active in sarcomas,
including ecteinascidine (ET743) and Glivec (STI571). This last drug has
been shown to be remarkably active in c-kit+ stromal sarcoma of the
gastro-intestinal tract. It can hopefully regarded as an example for
targeted therapies, which may come with a better understanding of the
molecular mechanisms triggered by the fundamental, specific genetic
alterations shown in sarcoma.
28
UI - 11847032
AU - Bergeron C; Ranchere-Vince D; Berard-Marec P
TI -
[Update on rhabdomyosarcomas in children]
SO - Bull Cancer 2002 Jan;89(1):108-12
AD - Departement de pediatrie, Centre Leon-Berard, 28, rue Laennec, 69373
Lyon Cedex 08.
Rhabdomyosarcoma is a rare tumour corresponding to 60-70% of soft tissue
sarcomas in children. Significant advances in treatment have been made
possible, and will be further obtained, by multicentric treatment
protocols conducted in paediatric oncology centres. Overall survival and
disease-free survival have been significantly improved over the past 30
years. In the meantime, diagnosis improvements have made the
classification of rhabdomyosarcomas more complex. A review of European
and American studies has evidenced a number of criteria that should be
taken into account for selecting treatment strategy: histological
examination (refined with the use of molecular biology) had proved very
informative, suggesting a worse prognosis for alveolar
rhabdomyosarcomas. Other criteria of interest are the tumour site
(favourable or unfavourable), patient age (under or above 10), tumour
size ( 5 cm), and disease stage. The number of sub-groups of patients
requiring different, more adapted treatment strategies increases with
the number of prognostic parameters to be considered. For convenient
clinical management, patients will therefore be classified into 4 risk
groups for systemic therapy (low, standard, high and very high risk),
whereas local treatment strategies will take into account the whole set
of prognostic criteria defined above.
29
UI - 11398160
AU - Wunder JS; Leitch K; Griffin AM; Davis AM; Bell RS
TI -
Comparison of two methods of reconstruction for primary malignant tumors
at the knee: a sequential cohort study.
SO - J Surg Oncol 2001 Jun;77(2):89-99; discussion 100
AD - University Musculoskeletal Oncology Unit, Mount Sinai Hospital, and the
Department of Surgery, University of Toronto, Toronto, Canada.
wunder@mshri.on.ca
BACKGROUND AND OBJECTIVES: The purpose of this study was to compare the
complications and functional outcome associated with the use of an
irradiated allograft-implant composite or a bone-ingrowth modular tumor
prosthesis for replacement of the knee joint after resection of a bone
sarcoma from the distal femur or proximal tibia. METHODS: Eleven
patients initially received an allograft reconstruction, followed by 64
treated with a tumor prosthesis. The primary analysis concerned
reconstructive failure, defined by the requirement for removal of the
original construct. Functional outcome was assessed by using the 1987
Musculoskeletal Tumor Society rating system. RESULTS: Reconstructive
failure occurred in 6 of 11 (55%) allograft constructs compared with 10
of 64 (16%) tumor prostheses (P = 0.009). Failures were due to infection
(2 of 11 allografts versus 4 of 64 prostheses; P = 0.2) or mechanical
complications (4 of 11 allograft fractures versus 5 of 64 broken
prosthetic stems and 1 aseptically loose prosthesis; P = 0.03). The limb
salvage rate was 95% (61 of 64) for patients with a tumor prosthesis
compared with 64% (7 of 11) for those with an allograft (P = 0.007).
Patients with a tumor prosthesis had a better functional outcome with a
mean score of 75% compared with 57% for those with an allograft
reconstruction (P = 0.006). CONCLUSIONS: This comparative study suggests
that limb salvage surgery at the knee has a better and more predictable
outcome with a tumor prosthesis than with an allograft-implant
reconstruction. Copyright 2001 Wiley-Liss, Inc.
30
UI - 11822026
AU - Shete S; Amos CI; Hwang SJ; Strong LC
TI -
Individual-specific liability groups in genetic linkage, with
applications to kindreds with Li-Fraumeni syndrome.
SO - Am J Hum Genet 2002 Mar;70(3):813-7
AD - Department of Epidemiology, The University of Texas M. D. Anderson
Cancer Center, Houston, TX 77030, USA. sshete@mdanderson.org
In this report, we present a simple and powerful way to incorporate
individual-specific liability classes into linkage analysis. The
proposed method is applicable to both quantitative and qualitative
traits. In linkage studies, we may have information about different
covariates. Incorpo
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